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What To Know About Primary Pulmonary Hypertension
Primary pulmonary hypertension, also called pulmonary arterial hypertension (PAH), occurs when arteries in the lungs become thick and narrow. This raises blood pressure in the lungs by restricting blood flow.
The condition also causes a person's heart to work harder to pump blood through the narrowed arteries. Eventually, a person's heart may lose the ability to effectively pump blood throughout the body.
PAH is a type of pulmonary hypertension, or high blood pressure in the lungs. Additionally, it is a rare, progressive disorder. This means it worsens over time. Treatments typically focus on managing symptoms and improving quality of life.
The condition most commonly affects females between the ages of 30 and 60, reports the American Lung Association. Healthcare professionals diagnose about 500–1,000 new cases of PAH in the United States each year, notes the association.
This article reviews the symptoms, causes, diagnosis, and treatment of PAH. It also discusses the potential outlook for someone with PAH.
PAH can cause several symptoms. They may appear at any stage in a person's life and can worsen over time.
At first, a person may not notice any symptoms, or they may believe the symptoms have another cause, such as other lung conditions.
As the disease progresses, a person may develop signs and symptoms that include:
The symptoms of PAH are nonspecific. This means several different diseases may cause the symptoms to appear. As a result, a doctor may misdiagnose the condition as other common lung diseases, such as chronic obstructive pulmonary disease (COPD) or asthma.
However, PAH is a rare condition. A person is more likely to have a condition like asthma or COPD than PAH.
Healthcare professionals do not know what exactly causes PAH. Other forms of pulmonary hypertension have underlying causes that include heart and lung diseases.
The condition may occur over time, following injury to the cells that line the blood vessels of the lung. If the cause of this injury is unknown, doctors refer to the condition as idiopathic PAH.
If healthcare professionals believe a genetic mutation inherited from a parent has led to the injury, doctors refer to the condition as heritable PAH. According to the American Lung Association, approximately 15–20% of people with PAH have heritable PAH.
Some other conditions associated with the development of PAH include:
Since there are similarities in symptoms between PAH and other common lung conditions, diagnosis can be challenging for healthcare professionals.
The diagnostic process typically involves eliminating other possible causes of a person's symptoms.
A healthcare professional will likely order several tests, which may include:
A doctor may also order genetic testing to check for genetic mutations associated with PAH. The presence of the condition in one or more family members can help a doctor confirm the diagnosis.
Delays in PAH diagnosis may occur for several reasons, including:
A person who has not received a PAH diagnosis should talk with a doctor if they experience:
There is no cure for PAH. Treatments focus on improving quality of life and slowing the progression of the disease.
Early diagnosis may help improve a person's outcome. Seeing a specialist at a pulmonary hypertension care center may help with getting a diagnosis and tailoring a treatment plan.
Some common treatment options include:
A person can also take steps to manage PAH at home, including:
People should speak with a doctor if they notice their symptoms worsening or changing. A doctor may be able to help a person find new therapies that work for them and suggest additional lifestyle strategies.
PAH does not have a cure. Medications, therapies, and lifestyle adjustments can help a person manage their condition long term.
Early diagnosis may help improve a person's survival. People may want to consider working with a specialist to help receive a prompt diagnosis and appropriate treatment.
A person may improve their outlook by following their treatment plan and taking steps to manage the condition at home.
PAH is a rare type of pulmonary hypertension. It may cause several, nonspecific symptoms at first. Doctors do not know exactly what causes the condition, but genetics may play a role in some cases.
Diagnosis may be challenging as the symptoms of PAH are similar to other lung conditions. Early treatment may improve a person's quality of life as well as slow disease progression.
A person should consider speaking with a healthcare professional if they experience any of the symptoms of PAH.
Pulmonary Hypertension Warning Signs: What To Look Out For
© Provided by Getty Images Pulmonary hypertension image Overview
A condition in which high blood pressure affects arteries of the lungs, and right side of the heart. This results in chest pain and shortness of breath.
Symptoms
If you or someone you know is exhibiting symptoms of a Pulmonary hypertension, seek medical attention immediately
The symptoms include
→ Common treatment options
→ How is this diagnosed?
Causes
There are several causes and may include:
→ Do you have a health question on your mind for pulmonary hypertension? Ask professionals from across the world
→ Interested to know more? Check out the full article here
What To Know About Portopulmonary Hypertension
Portopulmonary hypertension is increased arterial pressure in the vessels of the lungs and the vein that delivers blood to the liver.
Portopulmonary hypertension involves high blood pressure in the arteries in the lungs and the portal vein. The portal vein delivers blood from the intestines to the liver.
High blood pressure can develop separately in the lungs and portal vein. Doctors diagnose portopulmonary hypertension when both types of high blood pressure happen together.
Experts do not know exactly how many people live with this type of hypertension. Estimates suggest it accounts for anywhere from 5–15% of cases of high blood pressure in the lungs. It also affects about 5–6% of people with advanced liver disease.
This article covers the symptoms, causes, and treatments of portopulmonary hypertension.
Before understanding portopulmonary hypertension, it may be helpful to learn a bit about pulmonary hypertension and portal hypertension.
Pulmonary hypertensionDamage, scarring, or narrowing of the vessels in the lungs can cause an increase in blood pressure in the vessels of the lungs.
This type of high blood pressure is uncommon. Only about 500–1,000 people receive a pulmonary hypertension diagnosis in the United States each year.
Common causes of high blood pressure in the lungs include:
Portal hypertensionPortal hypertension involves high blood pressure in the portal venous system. The portal venous system delivers blood to the liver.
The most common cause of this type of high blood pressure is liver cirrhosis, also known as advanced liver disease.
Additional causes of portal hypertension include:
Doctors categorize the condition as portopulmonary hypertension when a person has high blood pressure in the portal vein and arteries of the lungs.
Portopulmonary hypertension tends to have a worse outlook and survival rate than pulmonary hypertension.
According to a 2020 review, a poor outlook may be more likely when a person has liver disease and portopulmonary hypertension.
Symptoms of portopulmonary hypertension are similar to the symptoms of pulmonary hypertension.
However, since it often develops due to liver disease, a person may also experience other symptoms, such as:
Portopulmonary hypertension often develops due to cirrhosis.
Over time, structural changes in the liver can cause alterations in the veins that may lead to problems with blood flow. This increases blood pressure in the portal veins, causing portal hypertension.
When portal hypertension develops, it also affects other areas of the body.
For example, the heart has to work harder, which may affect the vessels in the lungs, leading to portopulmonary hypertension.
In addition to advanced liver disease, the condition can also develop due to:
Factors that increase the risk of liver disease also increase the chances of developing portopulmonary hypertension. They include:
Doctors diagnose portopulmonary hypertension on the basis of a physical exam, symptom review, and diagnostic tests.
During a physical exam, doctors look for signs of leg swelling, heart murmur, and jugular vein distension (bulging of the major veins in the neck).
About 40 in 100 people with portopulmonary hypertension have a tricuspid valve murmur.
The standard diagnostic test to confirm a diagnosis of portopulmonary hypertension is a procedure known as right heart catheterization.
Diagnosis of portal hypertensionTo diagnose portal hypertension, doctors assess the clinical features of the condition using abdominal ultrasound and CT scans, including:
Treatment of portopulmonary hypertension remains similar to treating general pulmonary hypertension. The goal of treatment is to lower blood pressure in the arteries of the lungs.
Usually, treatment involves medications known as vasodilators to widen the blood vessels, which decreases blood pressure in the arteries.
Liver transplantation can help improve the condition. However, having portopulmonary hypertension may exclude a person from getting a liver transplant.
Portopulmonary hypertension is increased blood pressure in the portal veins that deliver blood to the liver and the pulmonary arteries in the lungs.
It typically develops due to severe liver disease.
Symptoms of portopulmonary hypertension include fatigue, chest pain, and shortness of breath, especially with activity.
Treatment involves medication that can help reduce pressure in the blood vessels.
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